ABSTRACT
Objective: Juvenile myasthenia gravis (JMG) is a rare disease with possible severe forms. Thymectomy is supported by many authors particularly in generalised forms with positive AcetylCholine Receptor Antibody (AChR- Ab). The aim of our study was to discuss the indication and the outcome of thymectomy in five children with JMG. Methods: We performed a retrospective study of the medical files of patients with JMG, who were hospitalised in our pediatric surgery department for thymectomy. For all the children we performed Acetylcholine Receptor Autoantibody (ACHRAB) test-system producer, a chest x-ray, a chest Computed Tomography Scan (CT-scan) and a Magnetic Resonance Imaging (MRI). Thymectomy was indicated on the presence of anomalies on CT-Scan or MRI and realised via either thoracotomy or video-assisted thoracoscopy. The study was conducted with the approval of the ethics committee at our institution. Results: Based on our findings, thymectomy in JMG with imaging anomalies seems to be associated with clinical improvement. In our patients complete remission after surgery was seen in one case, a clinical improvement with a decrease of the medical therapeutic dosage was noted in three other cases. One patient could not be evaluated because of non adherence to medical treatment. Surgery by thoracotomy or video assisted thoracoscopy (VAT) was also well tolerated by all the patients. Conclusion: The benefits of thymectomy in children with JMG are still controversial. However, many researchers have reported a sustained improvement of symptoms in the majority of patients after thymectomy. Our findings seem to encourage thymectomy in cases of JMG and particularly when imaging anomalies are found. VAT thymectomy was well tolerated and it is considered by many researchers to be the standard method for thymectomy in children.
ABSTRACT
Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period [3 days] for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors [even anicteric] have biliary cirrhosis, which requires lifelong follow up
ABSTRACT
Background: Neuroblastoma [NB] shows a complex combination of genetic aberrations. Some of them represent poor genetic prognosis factors that require specific and intensive chemotherapy. MYCN amplification consists of the major bad outcome prognostic factor, it is indeed frequently observed in aggressive neuroblastomas. To date different methods are used for MYCN status detection
Objectives: The primary aim of our study was to provide a critical assessment of MYCN status using 2 molecular techniques CISH and MLPA. We also focused on the correlation between neuroblastoma genetic markers and patient's clinical course among 15 Tunisian patients
Methods: we developed a descriptive study that includes 15 pediatric Tunisian patients referred to our laboratory from 2004 to 2011. We reported the analysis of fresh and FFPE NB tumors tissues
Results:No significant correlation was found between COG grade and patients overall survival. Assessment of NMYC gene copy number by kappa statistic test revealed high concordance between CISH and MLPA tests [kappa coefficient = 0.02]
Conclusion: Despite misdiagnosing of MYCN status fewer than 5 copies, MLPA remains an effective molecular technique that enables a large panel of genomic aberrations screening. Thus combining CISH and MLPA is an effective molecular approach adopted in our laboratory. Our results allow pediatric oncologists to set up the first Neuroblastoma therapeutic strategy based on molecular markers in Tunisia
ABSTRACT
Gastric perforation in neonates is an uncommon condition. It could be spontaneous but a contributing cause should be sought. To review our experience of treating 8 neonates with gastric perforation over the past 20 years. We reviewed the records of all newborns admitted to our hospital between 1990 and 2010 with regard to gender, age at admission, contributing factors, associated anomalies, site of perforation, type of operation, and outcome. Of the eight neonates, five were female and three male. The average weight was 2130 g. Four babies were premature. Three infants were ventilated for respiratory difficulty. Five patients had associated anomalies. Perforation occurred in the lesser curvature in 4, at the greater curvature in 3, and at the anterior antrum surface in 1. All patients were treated with gastrorrhaphy. Four neonates required additional gastrostomy. Mortality was 75% [6 infants]. Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation
Subject(s)
Humans , Male , Female , Rupture, Spontaneous/surgery , Retrospective Studies , Infant, Premature, Diseases/surgery , Infant, Newborn, Diseases , Gestational AgeABSTRACT
Since 1980s, the clinical and biological characteristics of urolithiasis in Tunisian children have continuously evolved. This retrospective study defines the current status of urolithiasis among children and adolescents in Tunisia. We retrospectively reviewed the records of 310 children and adolescents [age: 3 months - 19 years] between 2003 and 2010, holding urolithiasis. A first-line metabolic, urine and plasma work-up was performed in all patients. Physical and chemical analysis of the stones was performed respectively by stereomicroscopy and infrared spectroscopy. Statistical analysis of the results was performed with SPSS 11.0 software. The Chi-square test was used for comparison of percentages. Our study shows a male predominance of urolithiasis with a sex ratio of 1.5. Stones were located in the upper urinary tract in 70.7% of cases. Calcium oxalate was the predominant constituent in 52.6% of stones. There was an increasing prevalence of calcium oxalate stones according to age in both genders [48.6% in infants vs 68.5% in teenagers [P<0.01]]. Struvite was more frequent in patients aged 2-9 years [P<0.02] and significantly more prevalent in boys than in girls [P<0.001]. Ammonium urate stones were observed in 14.2% and were more frequent in infants. Our results emphasize a high percentage of calcium oxalate stones and a low percentage of struvite stones. The persistence of urate stones reflects the particular eating habits and the infectious risk factors. The patient's age is an important factor that must be taken into account during etiopathogenic work-up
ABSTRACT
The clinical and biological characteristics of urolithiasis in children are still evolving in our country. Since the eighties no study has been conducted to determine incidence of urolithiasis in Tunisia. To define the current status of urolithiasis inTunisian children. The records of 414 children with urolithiasis treated between 1983 and 2007 were found in a multi-centric study which took into consideration to age, sex, and history diagnosis and physical, laboratory, and radiologic findings. Clinical and biological date were performed in 344 cases. Stone analysis was performed by spectrophotometry. The incidence of paediatric urolithiasis has steadly decreased from 0.7/ 10 000 of the child population / year in 1991-1994 to 0.1 cases/10 000 children/ year in 2007. There was a continuous decrease in bladder stone over the past 25 years [47.1% in 1982-1986 Vs 10.2% in 2002-2007]. Stones were homogeneous in 31.4% of cases. The main component was calcium oxalate stones in 36.4% of the cases. Since the eighties the increase of oxalates stones [15.6% in eighties Vs 51.5% actually] were with depends to purines and struvite stones. Epidemiologic characteristics of the urinary lithiasis [sex, age, localization and composition] have changed in Tunisia, due to the improvements of standard of living. The occidentalization of the nutritional practices and the receding of the culinary traditions gave birth to new risks of urolithiasis characterized by the emergence of calcium oxalate stones
ABSTRACT
BACKGROUND: Studies that evaluate the effect of age on stone composition are scarce. The aim of this study was to highlight the changes in epidemiological characteristics (stone composition and location) of urolithiasis according to patients' age. METHODS: We studied 1,301 urolithiasis patients with age ranging from 6 months to 92 yr (781 males and 520 females). Stone analysis was performed using a stereomicroscope and infrared spectroscopy to determine the morphological type and molecular composition of each stone. RESULTS: The annual average incidence of new stone formation was 31.7 per 100,000 persons. In 71.8% of cases, calculi were located in the upper urinary tract. Compared to other age groups, children and old men were more affected by bladder stones. Calcium oxalate monohydrate was the most frequent stone component, even though its frequency decreased with age (59.5% in young adults and 43.7% in the elderly, P<0.05) in favor of an increase in uric acid stones (11.5% in young adults and 36.4% in the elderly, P<0.05). Struvite stones were rare (3.8%) and more frequent in children than in adults. CONCLUSIONS: The analysis of these data showed that urinary stones in Tunisian patients are tending to evolve in the same direction as the stones in patients from industrialized countries.